title: IgG4-related disease.
abstract: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated condition that can affect almost any organ and is now being recognized with increasing frequency. IgG4-RD is characterized by a lymphoplasmacytic infiltrate composed of IgG4(+) plasma cells, storiform fibrosis, obliterative phlebitis, and mild to moderate eosinophilia. The diagnosis of IgG4-RD unifies many eponymous fibroinflammatory conditions that had previously been thought to be confined to single organs. IgG4-RD lesions are infiltrated by T helper cells, which likely cause progressive fibrosis and organ damage. IgG4 antibodies are generally regarded as noninflammatory. Although autoreactive IgG4 antibodies are observed in IgG4-RD, there is no evidence that they are directly pathogenic. Rituximab-induced B cell depletion in IgG4-RD leads to rapid clinical and histological improvement accompanied by swift declines in serum IgG4 concentrations. Although IgG autoantibodies against various exocrine gland antigens have been described in IgG4-RD, whether they are members of the IgG4 subclass is unknown. The contribution of autoantibodies to IgG4-RD remains unclear.
authors.affiliation: Cancer Center.
authors.forename: Vinay S
authors.initials: VS
authors.lastname: Mahajan
authors.forename: Hamid
authors.initials: H
authors.lastname: Mattoo
authors.forename: Vikram
authors.initials: V
authors.lastname: Deshpande
authors.forename: Shiv S
authors.initials: SS
authors.lastname: Pillai
authors.forename: John H
authors.initials: JH
authors.lastname: Stone
mesh_terms: D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000097:blood / Q000276:immunology* / Q000473:pathology*; D005355:Fibrosis / Q000473:pathology; D006801:Humans; D006942:Hypergammaglobulinemia; D007074:Immunoglobulin G / Q000097:blood* / Q000276:immunology*; D007249:Inflammation / Q000097:blood / Q000276:immunology* / Q000473:pathology*; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology; D012698:Serologic Tests
chemical_list: D001323:Autoantibodies; D007074:Immunoglobulin G